Ophthalmologic disorders in children with syndromic and nonsyndromic hearing loss

To determine the rate of ophthalmologic anomalies among patients with syndromic and nonsyndromic, congenital sensorineural hearing loss (SNHL) to assess the need for comprehensive ophthalmologic evaluation in these children.

Retrospective medical chart review of children with SNHL who underwent comprehensive evaluation by pediatric ophthalmologists and geneticists.

Tertiary care pediatric hospital.

Seventy-seven patients with SNHL.

Degree of hearing loss (HL) and presence of ophthalmologic and genetic disorders.

The overall rate of ophthalmologic disorders was 32% (25 of 77 patients). When children with multisystem genetic disorders known to be related to visual loss were excluded, the rate fell to 23% (12 of 53 vs 13 of 24; P = .006). There was no statistically significant difference in the degree of HL between patients with and without eye disorders (mean [SD], 46.5 [29.9] vs 49.1 [32.3] dB HL; P = .75). Patients with eye disorders were significantly more likely to have a multisystem genetic disorder (13 of 25 [52%] vs 11 of 52 [21%]; P = .006). No patients with ocular abnormalities had isolated otologic disorders, but 9 of 52 (17%) of those patients without ocular abnormalities did.

Comprehensive ophthalmologic examination revealed a rate of ophthalmologic disorders in children with SNHL in the lower end of the previously reported rates of 31% to 61%. Children with nonsyndromic SNHL have an approximately 2- to 3-fold increase in ocular abnormalities compared with the general pediatric population. Ophthalmologic and genetic consultations are warranted in patients with congenital SNHL.